PANRE Hematology 2

Question 1

A 55-year-old male presents with plethora, headache, and pruritus after bathing. Hgb is 19.4 g/dL, Hct 58%, WBC 13,000/μL, platelets 520,000/μL. JAK2 V617F mutation is positive. What is the most likely diagnosis?

Accepted Answer

Polycythemia vera

Answer

Polycythemia vera is a myeloproliferative neoplasm characterized by elevated red cell mass, JAK2 V617F mutation, and aquagenic pruritus, with associated thrombocytosis and leukocytosis.

Question 2

A 24-year-old African American male develops hemolytic anemia and hemoglobinuria after being prescribed dapsone for dermatitis herpetiformis. Peripheral smear shows Heinz bodies. What is the underlying defect?

Accepted Answer

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Answer

G6PD deficiency causes oxidative hemolysis when triggered by oxidant drugs (dapsone, primaquine), infections, or fava beans, resulting in Heinz body formation and intravascular hemolysis.

Question 3

A 6-year-old boy has recurrent hemarthroses and prolonged bleeding after minor trauma. PTT is 68 seconds (normal <35), PT is normal, platelet count is normal, bleeding time is normal. Factor VIII activity is 2%. What is the diagnosis?

Accepted Answer

Hemophilia A

Answer

Hemophilia A is caused by factor VIII deficiency, presenting with hemarthroses and deep muscle bleeds, prolonged PTT with normal PT, and very low factor VIII activity levels.

Question 4

A 30-year-old presents with pancytopenia: Hgb 7.1, WBC 1,800, platelets 28,000. Bone marrow biopsy shows a hypocellular marrow with >70% fat cells. No splenomegaly. What is the most appropriate treatment for severe aplastic anemia in a patient with a matched sibling donor?

Accepted Answer

Allogeneic hematopoietic stem cell transplantation

Answer

Allogeneic hematopoietic stem cell transplantation from a matched sibling donor is the curative treatment of choice for severe aplastic anemia in young patients.

Question 5

A patient has a positive direct Coombs test, elevated indirect bilirubin, low haptoglobin, and reticulocytosis. Peripheral smear shows spherocytes. Splenomegaly is present. What condition is most consistent with these findings?

Accepted Answer

Autoimmune hemolytic anemia

Answer

Autoimmune hemolytic anemia (AIHA) is confirmed by a positive direct Coombs (DAT) test, which detects IgG or complement on RBC surfaces, causing extravascular hemolysis with spherocyte formation.

PANRE Test Practice Test

PANRE Test Practice Test

PANRE Hematology 2